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Congenital Heart Disease (Part I)

Shenzhou Hospital
He Lihui

Overview
Congenital heart defects are abnormalities in the heart's structure that are present at birth.
Approximately 8 out of every 1,000 newborns have congenital heart defects, ranging from mild to severe.

Etiology
1.Genetic factor (internalfactor) chromosomal aberrations :Genetic and

2. Environmental factor (external factor): High altitude 3. Other related factors: Viral infections of pregnancy , Mothers who are diabetic, alcoholics or drug addictive Drugs and metabolic factors

4. Inherited factor

Prevention
The health protection of pregnant woman should be enhanced. High risk factors, such as drugs, radiation, viral infection, et.should be avoided. Suit dosage Folic Acid should be filled up in early pregnancy stage.

Evaluating a child with a heart murmur


Does the child have heart disease? Is it congenital heart disease? If it is congenital heart disease, what is the lesion? What is the severity of the lesion?

Assessment of a child for the presence of heart disease


Major 1. Systolic murmur garde III or more specially with a thrill 2. Diastolic murmur 3. Cyanosis 4. Congestive heart failure Minor 1.Systolic murmur less than grade III in intensity 2. Abnormal S2 3.Abnormal ECG 4.Abnormal X-ray 5.Abnormal BP

CHD

Acyanotic CHD

Cyanotic CHD

L to R shunts ASD VSD PDA

Obstructive lesions Pulmonic stenosis Aortic stenosis Coarctation of aorta Mitral regurgitation

R to L shunts
TOF Complete TGA

Feature of the shunts


Left to right shunts
Generally no cyanosis Increased pulmonary circulation Decreased systemic circulation

Right to left shunts


Cyanosis appears early Pulmonary circulation increase or decrease Deoxygenated blood mix with oxygenated blood in systemic circulation

Pulmonary arterial hypertention (hyperkineticobstructive) Persistent cyanosis in late stage (Eissenmagers syndrome)

Atrial Septal Defect


ASD

Definition:
ASD is an abnormal communication between the two atria.

Classification:
Ostium secundum type

Ostium primum type


Endocardial cushion type

ASD
Accounts about 5%~10% of all CHD cases.
The incidence is estimated to be 1 per 1500 live births. Is the most common CHD in adult.

Male : Female 1: 2

PV

SVC
RA AO

PV LA
PA LV

IVC RV
ASD murmu r

Hemodynamics Figure of ASD

Pathophysiology of ASD LR shunt determined by

Size of the ASD lesion Pressure difference between two atria RV diastolic accommodation

Hemodymamics of ASD
Oxygenated blood in PVs LA ASD shunting SVC IVC RA enlargement

LV Aorta Ejection Systemic circulation insufficience

RV enlargement

PA congestive

Pul. Circulation congestive

Pul. Arterial Hypertension

Obstructive PAH
Eisenmangers syndrome

Pre Obstructive PA Hypertension


SVC IVC
Shunting Hypertrophy RA Hypertrophy RV Volume of blood Pul. ArteryDilated LA LV ( Ejection of Blood AortaBlood

Pulmonary Vein

Pul. Circulation ( Congestive Sys. Circulation Insufficiency

Frequent Chest Infection Congestive CHF

Failure To Thrive

Post Obstructive PA Hypertension


SVC IVC Shunting Hypertrophy RA Hypertrophy RV Pul. ArteryDilated Hyperkinetic PA Hypertension Sys. Circulation (Mixed Blood LA LVMixed Blood Pul. Vein

Obstructive PA Hypertension
Cyanosis Eisenmangers Syndrome

Clinical findings of ASD


Symptoms

Generally asymptomatic
Pulmonary plethoric: frequent chest infections Systemic Circulation InsufficiencyFailure to thrivepoor weight gainfeeding difficulty fatigue shortness of breathesweating CyanosisSevere cyanosis in large lesions softer heart murmur and accentuated P2.

sound: S1 accentuated - loud S2 widely split and fixed( Volume in RV Prolonged ejection phase-Pul.Valve closes late P2 accentuated Murmurs Shunt Murmur: Absent Flow Murmurs: (a) A grade -/ ejection systolic murmur is heard best at LSB2-3 which widely transmitted all over the chest. No thrill. (relative Pul. Valve stenosis)
(b) Delayed Diastolic Murmur at LLSB ( relative Tricuspid sterno

Sign of ASD

Complication of ASD

Bronchopneumonia Congestive heart failure Infective endocarditis

ECG of ASD
Right Ventricle HypertrophyRVH) Right Axis Deviation Incompleted right bundle branch block (IRBBB)I0

X-ray findings
Plethoric Lung fields RA and RV enlargement Prominent PA segment Normal or small aortic shadow

USG findings
RA , RV enlargement RV overloaded Parallel shunt between atria in Doppler

Catheterization
1SaO2 in RAin Vena Cava 2Pressure of RV and PA is mormal or mildly 3Catheter passing through the lesion can enter RV from RA.

Prognosis and treatment of ASD


Prognosis: Lesions diameter<3mm mostly closed within 3months of age. Lesions diameter>8mm rarely close without any intervention.
Large shuntQp/Qs>1.5needs operation. Invasive cardiac catheterization Amplazercardia seal ect. device to seal the lesion

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